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INFLAMMATORY OPTIC NEURITIS & NEURORETINITIS

Dr. Vishal Kulkarni MS ( Ophthal)

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Optic Neuritis
Background: historically
Blindness a divine punishment for sin Optic neuritis may have accounted for miraculous spontaneous cures

Defination
Inflammation of optic nerve is known as optic neuritis , Characterized by sudden loss of vision in affected eye, often accompanied by pain

Types Demyelinating ON - by far the most common cause of ON .

Non-Demyelinating ON Parainfectious ON Infectious ON Autoimmune ON -

Aetiology of optic neuritis

Immune-mediated disorders

Demylinating disorders
Isolated

Local
Uveitis Sympathetic

ophthalmitis

As. with Multiple Sclerosis

Neuromylitis optica

Systemic Sarcoidosis,wegeners

granulomatosis,Acute disseminated encephaalomylietis

Idiopathic Infectious optic neuritis

Syphilis Sinus-related Cat-scratch Fever (Bartonellosis) LymeDisease (Borreliosis In HIV / Immunocompromisedindividuals, TB Herpes Zoster Toxoplasmosis Toxocariasis CMV Cryptococcus

Local Endopthalmitis .Orbital cellulitis ,Sinusitis ,Contagenous spread

Epidemology
Incidence -3-5 /lakh per year Prevalence -115 per 1lakh Majority of patients are between the age of 20-50 yr Females are more affected than males Acute demyelinating is the most common form of optic neuritis May be the initial presentation of Multiple Sclerosis

Ocular manifestations
Visual acuty -Subtle or profound visual loss
,usually unilateral may be b/l

Contrast sensitivity Stereoacuity Pain aggravated by eye movements -Deep orbital

,retro orbital or brow

Illsustained pupillary reactions Visual field defects:

Central 30 focal central/cecocentral scotomas

VEP
Prolonged latency is seen

Dyschromatopsia

(loss of colour vision usually red desaturation)

Pulfrich phenomenon Pulfrich phenomenon altered perception of moving objects

Uhthoffs sign 50% of cases of ON

Active or recovered

Transient obscuration of vision with body temp

Exercise Hot bath/shower Hot weather

Ophthalmoscopic types-Papillitis
Approx 35 % cases of optic neuritis Inflammation is located anterior to nerve First hyperemic disc , then blurred margins , swelling & edema ,torturous & distorted veins Disc swelling does not exceed 2-3 D colour vision, contrast sensitivity ,stereo acuity diminished . Central Vn spared

Post neuritic atrophy

Dirty grey colour disc Filled with organised tissue extending on consticted arteries as perivascular shething

Neuroretinitis
Entity characterized by optic disc edema & macular star(ODEMS). 3-4 th decade of age. Sudden decrease in vision not associated with pain Vision ranges from PL- 6/9 RAPD Oedma precedes macular star , which may not be presnt at the presentation

Neuroretinitis
Etiology Papilitis with retinal exudates which are in retinal nerve fiber layer & radially oriented to form macular fan or star .
50% cases are idiopathic Following a viral syndrome 2/3rd of neuroretinitis Cat Sratch Disease Toxoplasmosis Toxocariasis and helminths . Lyme disease, Syphilis, especially secondary syphilis as part of a meningitis

Investigations
Every case of neuro retinitis should be investigated for infectious etiology
Routine haemogram, X-ray (Chest), Mantoux, FTA-ABS & VDRL for syphilis, serology and culture for bartonella Serology for toxoplasmosis Blood culture

FA in neuroretinitis

Fig a b c -Early hyperflurence and late leakage on the disc

On FA leakage of dyes at disc not on macula

Treatment
No treatment is also option as there is spontaneously But treatment may speed recovery & shorten duration

Specific antibiotic therapy

Idiopathic cases are presumed to be viral ,- No treatment required Bartonella Doxycycline Syphilis iv Penicillin

Acute retrobulbar neuritis

Neither the patient nor the ophthalmologist sees anything
No ophthalmoscopically visual changes Occasional temopral pallor seen If lesion is near lamina cribrosa some signs of papilitis with distension of veins & attenuation of arteries

Acute retrobulbar neuritis

C/f are essentially similar to acute pappilitis except for more marked pain during ocular movements ,& fundus appears normal. Presence of RBN always arouse suspicion of presence of multiple scerosis. MRI brain is recommended in every case of RBN

Diagnosis

Visual acuity

Colour vision
Fundus

Stereoacuity
Contrast sensitivity

Visual fields
Central scotomas

Paracentral scotomas

Altitudinal defects

Role of CSF analysis:

Elevation of Ig G Elevation Ig G / albumin index ,presence of oligoclonal Ig G bands Seen in about 90% MS

Diagnosis & testing

MRI FLAIR sequencing Gadolinum enhancement

Optic nerve sheath enhancement with Gad Periventricular whire matter lesion on MRI

 To determine whether the cause is compressive inflammatory , infectious . To determine prognosis or risk for subsequent development of MS in mono symptomatic cases. To confirm the diagnosis & to access the other white matter lesion

MRI-White matter lesions

Fluorescein Angiography-Papillitis

OCT
Predicting the subset of optic neuritis patients who will suffer from persistsant visual disfunction Poor visual outcome who had RNFL thickness less than 75 um in 3-6 months duration .

Left: OCT-showing average retinal nerve fiber layer measurements in affected eye at time of presentation, measuring 111 m in right eye (top) and 99 m in left eye (bottom). Right: thinning of average retinal nerve fiber layer in right eye (top), which now measured 44 m 6 months after acute optic neuritis event.

Typical optic neuritis

Predominantly affects females 5:1
Retroocular pain made worse with eye movements Common in the age group of 15 to 45 Uniocular Peak visual loss within 2 week

Atypical optic neuritis

No gender predilection
Painless visual loss

Visual loss may be subtle to complete Starts improving thereafter

Old patients Bilateral Progression of visual loss beyond 2 week Patients fail to improve with treatment Disc haemorrhages and cotton wool spots can occur. spots can occur

Systemic Investigation
Routine hemogram X-ray (Chest) Mantoux FTA-ABS & VDRL for syphilis serology and culture for bartonella Serology for toxoplasmosis Markers of viral infection Serum electrolytes and FBS MRI Lumbar puncture and CSF tap (IgG index and oligoclonal bands) Blood culture ANA,dsDNA

AION Age Pain Pupil Defect Optic disc >50 yr Unsual +RAPD Altitudinal

Optic Neuritis <40 yr With eye movements 92% + RAPD Central

Edema 100% may Edema 33 % hyperemic be pale Common Delayed disc filling No optic nerve enhansment Unsual No delayed disc filling

Retinal Hge FFA

MRI scan

Optic nerve enhancement

Differentiating points between Papilloedema and optic neuritis

Disc edema Visual acuity Color vision and contrast RAPD MRI

Papilloedema Present

Optic Neuritis Present in 30%

Normal till late stages despite sever disc edema Normal till late stages

Usually decreased

Severely decreased

Present only if optic Always present atrophy sets in Shows demyelinative MRI Shows lesions in periventricular causative lesion ventricles region and enlargement

Optic neuritis in MS
Unless otherwise specified .the term optic neuritis implies an idiopathic or demylinating syndrome associated with multiple sclerosis Optic Neuritis occurs in about 50% of patients with MS Is presenting feature in about 20%. Occurrence of retro bulbar neuritis should always arouse suspicion of multiple sclerosis ONTT has shown that 10-year risk of development of multiple sclerosis is 38%.
Typical lesion s on MRI One or more typical lesions Risk of developing MS 56%

No lesions

22%

Development of multiple sclerosis.

Positive risk factors
female gender, one or more brain lesions seen on MRI, h/o non specific neurological symptoms (usually transient numbness ), prior optic neuritis in fellow eye or Retro bulbar ON Negative risk factors Male gender, no lesions on MRI, optic disc swelling, absence of pain and ophthalmoscopic findings

Pathophysiology
Autoreactive abs & T-Cells cross blood-brain barrier & damage myelin demyelination Genetic & environmental factors predispose to an autoimmune response
Genetic: HLA-Dw2; HLA-DR2 Environmental: Infection, stress, systemic antigens & metabolites

Differential diagnosis
Lebers optic neuropathy lack of pain, circumpapillary telangiectatic microangiopathy, absence of leak on FA & maternal pattern of inheritance.

Figure 1 Acute fundal appearance in Leber hereditary optic neuropathy showing disc hyperaemia, swelling of the parapapillary retinal nerve fibre layer and retinal vascular tortuosity

Sarcoidosis
Granulomatous inflammation of optic nerve Disc has characteristic lumpy white appearance ,d/t granulomatous inflammation . Rapid recovery with corticosteroid therapy but recurrence on steroid tapering is characteristic Which is unusual for demylinating optic neuritis

syphilis
U/L or B/L Vitreous cellular reaction is typical feature to diffn from demylinationg ON where vitreous humor is usually clear CSF VDRL iv Penicillin

Cat Scratch disease

B.Hensele. Most commen cause of neuroretinitis 1-2% in pts of CSD

Neuroretinitis with macular star

 Diffuse disc edema ,nerve fiber layer hamorrage , cotton wool spots ,stellate macullar exudates

Treatment Infection usually selflimiting May be treated with oral doxycycline or erythromycin, with or without rifampicin when infections are severe or sight threatening1

toxoplasmosis
Most important protozoan cause of intraocular inflammation Retinochoroiditis ,pappilitis , neuroretinitis, Focal necrotising retinitis , atropic scars Oval or circular lesions over cream coloured retina, retinal thickening

Optic Neuritis Treatment Trial

15 centers in the U.S. (1988-92)

457 pts: acute unilateral ON & MS 18-46 yrs of age; 77% ; 85% caucasian
3 treatment groups: (1) IV methyl prednisolone 250mg Q6hrs x 3 days 11 days Oral prednisone (1mg/kg) (2) Oral prednisone (1mg/kg) x 14 days (3) Placebo Baseline gadolinium-enhanced MRI of brain/orbits 1 visual outcome measures: visual acuity, color vision, contrast sensitivity & visual field; 2 outcome measure: development of CDMS

Recommendations
IV methyl prednisolone dose of 250 mg IV slowly over 30-60minutes repeated for 6 hrs for 3 days , Followed by 11 days of oral prednisolone ,1 mg/ kg /day

Never use oral prednisolone alone ,as it may recurrence rate are significantly higher
If MS is already diagnosed or suffered from prior episode of optic neuritis ---- observation is rule .

CHAMPS:
Objectives

Controlled High-risk Subjects Avonex Multiple Sclerosis

Conclusions after 3 yrs Group 2

Group 1
Whether interferon

beta la (Avonex)
treatment would benefit patients who had experienced a first acute demyelinating event involving the optic nerve, brain stem/cerebellum, or spinal cord, and who displayed MRI brain abnormalities

Interferon beta 1-A onceweekly IM inj

Placebo injections
e

CDMS is 50 % in placebo group 35% in interferon group

Future neurological events Worsoning of brain MRI Gadolinum enhansed lesion

Early Treatment Of Multiple sclerosis Study

Study objective: to determine whether an early treatment with interferon b 1a is effective in delaying the development of CDMS after the first attack. Treatment - IFN beta-1a 22 mcg SC weekly or Placebo ,followed for 2 years.

ETOMS

Champions Study
Controlled High Risk Avonex Multiple Sclerosis Prevention Surveillance Objective: Compared outcomes in those who had given drug from the start of the CHAMPS study (Immediate treatment or IT group) versus those who had switched from placebo after about 30 months (delayed treatment or DT group). Results: IT group had significantly fewer relapses and fewer MRI brain lesions than the DT group.

Contribution from RPC

Objective- A pilot study in RPC showed dexamethasone to be equally effective and a cheaper alternative to methyl prednisolone in treating optic neuritis. Treatment ;- with intravenous dexamethasone (100 mg in 250 ml of 5% dextrose over 1-2 hours daily, for3 days). Conclusions are promising .

Newer modalities ; Copolymer-1 T-Cell Receptor Peptide Immunization Anti-CD4 Monoclonal Antibody Azathioprine (Imuran) Cyclophosphamide (Cytoxan) Oral Myelin Methotrexate Cladribine Intravenous Immunoglobulin G

th

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